Osteogenesis imperfecta (OI) is a rare genetic disorder of increased bone fragility and low bone mass. It is conceivable that children and adolescents with OI are less active than healthy peers because of frequent fractures, immobilization,functionals limitations and no adapted physicals activity(APA). The hypothesis is that an Adapted physique activity could improve access of activity for patients with Osteogenesis Imperfecta (OI). The aim of the study is to evaluate benefice of APA,improve aerobic capacity, cardiovascular and bone benefits, and gain of quality of life. Children with OI between 6 and 18 years old will have a program of supervised "adapted training program" during one year. The program is adapted at each individual and without risk for the patient.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
PREVENTION
Masking
NONE
Enrollment
30
Adapted sports practices for 30 minutes twice a week
Centre de Référence des Maladies Rénales Rares - Hospices Civils de Lyon - Service de Néphrologie et Rhumatologie Pédiatriques - Hôpital Femme Mère Enfant
Bron, France
6 minutes walk test
Distance traveled (in meters) in 6 minutes
Time frame: Month 0
6 minutes walk test
Distance traveled (in meters) in 6 minutes
Time frame: Month 6
6 minutes walk test
Distance traveled (in meters) in 6 minutes
Time frame: Month 12
Weight
weight in kilograms will be measured with a scale
Time frame: Month 0
Weight
weight in kilograms will be measured with a scale
Time frame: Month 6
Weight
weight in kilograms will be measured with a scale
Time frame: Month 12
Height
Size in meter measured with a meter
Time frame: Month 0
Height
Size in meter measured with a meter
Time frame: Month 6
Height
Size in meter measured with a meter
Time frame: Month 12
body mass index
calculated with weight and height
Time frame: Month 0
body mass index
calculated with weight and height
Time frame: Month 6
body mass index
calculated with weight and height
Time frame: Month 12
blood pressure
measurement of blood pressure (cm Hg)
Time frame: Month 0
blood pressure
measurement of blood pressure (cm Hg)
Time frame: Month 6
blood pressure
measurement of blood pressure (cm Hg)
Time frame: Month 12
phosphocalcic biologic parameters
Ionosodium (mmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
potassium (mmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
calcium (mmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
chlore (mmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
bicarbonates (mmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
glucose (mmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
urea (mmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
creatinemia (mmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
phosphorus (mmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
proteinemia (g/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
Vitamin D (nmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
1-25 OH vitamin D (pmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
PTH (ng/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
alkaline bone phosphatases (U/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
CTX (pg/ml)crosslaps
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
osteocalcin (µg/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
ferritin (µg/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
blood count formula : leucocytes (giga/L), erythrocytes (tera/L), hemoglobin (g/L), hematocrit (%), platelets (giga/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
calciuria (mmol/L)
Time frame: Month 0 and Month 12
phosphocalcic biologic parameters
Ionosodium (mmol/L) potassium (mmol/L), calcium (mmol/L), chlore (mmol/L), bicarbonates (mmol/L), glucose (mmol/L), urea (mmol/L), creatinemia (mmol/L), phosphorus (mmol/L), proteinemia (g/L), Vitamin D (nmol/L), 1-25 OH vitamin D (pmol/L), PTH (ng/L), alkaline bone phosphatases (U/L), CTX (pg/ml)crosslaps, osteocalcin (µg/L), NFS, ferritin (µg/L), blood count formula : leucocytes (giga/L), erythrocytes (tera/L), hemoglobin (g/L), hematocrit (%), platelets (giga/L), calciuria (mmol/L), urinary creatinin (mmol/L).
Time frame: Month 0 and Month 12
ration lean mass / fat mass
Dual-energy X-ray absorptiometry (DXA)
Time frame: Month 0
ration lean mass / fat mass
Dual-energy X-ray absorptiometry (DXA)
Time frame: Month 12
Respiratory Functional Exploration
Time frame: Month 0
Respiratory Functional Exploration
Time frame: Month 12
Thickness intima / media
echography
Time frame: Month 0
Parameters of the connected watch : number of steps
data recovery of the connected watch
Time frame: Month 0 and Month 6 and Month 12
Parameters of the connected watch : distance traveled
data recovery of the connected watch
Time frame: Month 0 and Month 6 and Month 12
Parameters of the connected watch : calories consumption
data recovery of the connected watch
Time frame: Month 0 and Month 6 and Month 12
Ratio thickness intima / media
echography
Time frame: Month 12
Bone mineral density
Dual-energy X-ray absorptiometry (DXA)
Time frame: Month 0
Bone mineral density
Dual-energy X-ray absorptiometry (DXA)
Time frame: Month 12
Number of new fractures
Time frame: Month 1
Number of new fractures
Time frame: Month 3
Number of new fractures
Time frame: Month 6
Number of new fractures
Time frame: Month 9
Number of new fractures
Time frame: Month 12
Quality of life questionnaire : PedsQL
Measurement Model for the Pedratric Quality of Life Inventory The 4 Multidimensional Scales and 3 Summary Scores are Scales Physical Functioning (8 items) Emotional Functioning (5 items) Social Functioning (5 items) School Functioning (5 items) Summary Scores Total Scale Score (23 items) Physical Health Summary Score (8 items) Psychosocial Health Summary Score (15 items)
Time frame: Month 0
Quality of life questionnaire : PedsQL
Measurement Model for the Pedratric Quality of Life Inventory The 4 Multidimensional Scales and 3 Summary Scores are Scales Physical Functioning (8 items) Emotional Functioning (5 items) Social Functioning (5 items) School Functioning (5 items) Summary Scores Total Scale Score (23 items) Physical Health Summary Score (8 items) Psychosocial Health Summary Score (15 items)
Time frame: Month 6
Quality of life questionnaire : PedsQL
Measurement Model for the Pedratric Quality of Life Inventory The 4 Multidimensional Scales and 3 Summary Scores are Scales Physical Functioning (8 items) Emotional Functioning (5 items) Social Functioning (5 items) School Functioning (5 items) Summary Scores Total Scale Score (23 items) Physical Health Summary Score (8 items) Psychosocial Health Summary Score (15 items)
Time frame: Month 12
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